Dataset Information

Unexpected presentation of a type IV laryngo-tracheo-oesophageal cleft: Anaesthetic implications of a rare case.

ABSTRACT: Laryngo-tracheo-oesophageal cleft (LTEC) is a congenital midline defect of the posterior larynx and trachea and the anterior wall of the oesophagus. Existence of these clefts may not be apparent during pre-operative evaluation. We present a rare case of a neonate initially scheduled for tracheo-oesophageal fistula repair. Unexplained air leak in spite of placement of size 4.0 mm endotracheal tube in a 2.5 kg neonate triggered extensive intra-operative evaluation of the airway anatomy via flexible and rigid bronchoscope. A type IV LTEC with an unusual anatomy was identified that was considered surgically irreparable. This case also highlights the need for a team approach and preparedness of anaesthesiologists for a difficult airway while managing such cases. Maintenance of high degree suspicion is warranted.

SUBMITTER: Dwarakanath S

PROVIDER: S-EPMC4296363 | biostudies-literature | 2014 Nov-Dec

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Unexpected presentation of a type IV laryngo-tracheo-oesophageal cleft: Anaesthetic implications of a rare case.

Dwarakanath Sanjay S Reddy Arundathi A

Indian journal of anaesthesia 20141101 6

Laryngo-tracheo-oesophageal cleft (LTEC) is a congenital midline defect of the posterior larynx and trachea and the anterior wall of the oesophagus. Existence of these clefts may not be apparent during pre-operative evaluation. We present a rare case of a neonate initially scheduled for tracheo-oesophageal fistula repair. Unexplained air leak in spite of placement of size 4.0 mm endotracheal tube in a 2.5 kg neonate triggered extensive intra-operative evaluation of the airway anatomy via flexibl ...[more]

PMID: 25624542

Similar Datasets

Project description:A laryngo-tracheo-esophageal cleft (LC) is a congenital malformation characterized by an abnormal, posterior, sagittal communication between the larynx and the pharynx, possibly extending downward between the trachea and the esophagus. The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx. These incidence rates may however be underestimated due to difficulty in diagnosing minor forms and a high mortality rate in severe forms. A slightly higher incidence has been reported in boys than in girls. No specific geographic distribution has been found. Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress. Five types of laryngo-tracheo-esophageal cleft have been described based on the downward extension of the cleft, which typically correlates with the severity of symptoms: Type 0 laryngo-tracheo-esophageal cleft to Type 4 laryngo-tracheo-esophageal cleft. LC is often associated with other congenital abnormalities/anomalies (16% to 68%), mainly involving the gastro-intestinal tract, which include laryngomalacia, tracheo-bronchial dyskinesia, tracheo-bronchomalacia (mostly in types 3 and 4), and gastro-esophageal reflux disease (GERD). The syndromes most frequently associated with an LC are Opitz/BBB syndrome, Pallister Hall syndrome, VACTERL/VATER association, and CHARGE syndrome. Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum. The causes of the embryological developmental anomalies leading to LC are not known but are thought to be multifactorial. LC appears to be mostly sporadic although some familial cases with suspected autosomal dominant transmission have been reported. The age of diagnosis depends mainly on the severity of the clinical symptoms and therefore on the extent of the LC. Diagnosis is made either based on clinical manifestations or on investigations, such as endoscopy, X-ray, CT scan, performed for other conditions. Differential diagnoses include tracheo-bronchial fistula, gastro-esophageal reflux disease and neurological swallowing disorders, as well as laryngomalacia and laryngeal palsy. Prenatal diagnosis of LC has never been reported, although associated anomalies may be detected on fetal ultrasonography. Once the cleft is diagnosed, it is essential to determine its length to orient the management and treatment approach. Management involves maintenance of satisfactory ventilation, prevention of secondary pulmonary complications as a result of repeated aspirations, and adequate feeding. Endotracheal intubation may be required for respiratory distress in severe cases. Treatment requires endoscopic or external surgery to close the cleft. Surgery should be performed as early as possible to avoid complications related to aspiration and gastric reflux, except in type 0 and type 1 cases in which conservative measures must first be attempted. The prognosis is variable depending on the severity of the LC and associated malformations. Early diagnosis and appropriate treatment and management help to reduce mortality and morbidity.

Project description:PurposeThe COVID-19 pandemic has resulted in a global health crisis of unparalleled magnitude. The direct risk to the health of children is low. However, disease-containment measures have society-wide impacts. This study explored the pandemic experiences of parents of children with oesophageal atresia/tracheo-oesophageal fistula (OA/TOF) in the UK.DesignA phenomenological approach underpinned use of an asynchronous online forum method, in collaboration with a patient support group. Data were evaluated using thematic analysis.ResultsThe online forum ran between 7 November and 18 December 2020 with 109 participants.Pandemic experiences were divided into themes relating to healthcare and disease containment. Participants described positive experiences with remote healthcare but identified limitations. Delays and cancellations led to escalation of care to an emergency level, slower developmental progress and feelings of being abandoned by services. Inpatient care was perceived as safe but caring alone was emotionally and practically challenging. Disease containment themes revealed anxiety regarding health risks, 'collateral' damage to well-being because of isolation, and an impact on finances and employment. Parents described a transition from worry about direct health risks to concern about the impact of isolation on socialisation and development. A process of risk-benefit analysis led some to transition to a more 'normal life', while others continued to isolate. Benefits to their child's health from isolation were reported.ConclusionsParents' experiences of caring for a child with OA/TOF during the pandemic were varied. Rapid adoption of telehealth has demonstrated the enormous potential of remote healthcare delivery but requires refinement to meet the needs of the individual. Future pandemic planning should aim to retain community healthcare services to avoid escalation of care to an emergency, manage chronic and developmental concerns, and support parental well-being. Accurate and consistent disease-specific information is highly valued by parents. Third sector organisations are ideally positioned to support this.

Dataset Information

Unexpected presentation of a type IV laryngo-tracheo-oesophageal cleft: Anaesthetic implications of a rare case.

Publications

Unexpected presentation of a type IV laryngo-tracheo-oesophageal cleft: Anaesthetic implications of a rare case.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets